Case Of Intravenous Leiomyomatosis Research Articles

Surgical treatment of intravenous leiomyomatosis with intracardial growth in conditions of artificial circulation. Clinical case

Intravenous leiomyomatosis is a smooth muscle tumor of unclear malignant potential capable of intravascular growth with varying levels of intravascular advancement. The only radical treatment method is surgical intervention requiring quality preoperative preparation and determination of optimal surgical tactics taking into account the level of damage. A description of a clinical case of intravenous leiomyomatosis with intracardial growth is presented

A case of intravenous leiomyomatosis with intracardiac extension.

A case of intravenous leiomyomatosis with intracardiac extension.

Intravenous leiomyomatosis of the uterus: still discovered on anatomopathological examination

Background: Leiomyomas beyond the uterus are defined by benign smooth muscle cell tumors outside of the uterus. Intravenous leiomyomatosis is a rare type of uterine leiomyoma and is characterized by the formation and growth of benign leiomyoma tissue within the vascular wall. Herein, we present a case of Intravenous leiomyomatosis successfully treated by surgical removal and a review of actual medical recommendations. Case presentation: A 49 - year-old woman, maghrébin, G3 P2, no family history of uterine myomas mentioned, having systemic arterial hypertension, presented to our department with hypogastric pain and abnormal uterine bleeding in the prior five months resulting in anemia which required iron supplementation. On physical examination the vital signs were normal. A palpable mass in the hypogastrium was noted. The rest of the exam was unremarkable. Pelvic ultrasound showed a huge uterus with multiple heterogeneous leiomyomas, including at least one intracavity. Computed tomography scans and magnetic resonance imaging were not done initially due to the unaffordability of the patient. The initial diagnosis was leiomyoma. The decision to perform a total abdominal hysterectomy and bilateral salpingo-oophorectomy was taken. The abdomen was opened by a midline vertical incision. During surgery, multiple subserosal, intramural and submucosal fibroids ranging from 2 cm × 3 cm to 10 cm × 10 cm were seen. On pathological examination, the uterus measured 19 cm in the largest diameter and weighed 1.3 kg. The cut section showed white nodular myometrial masses. Microscopically, intravascular growth of benign smooth muscle cells is found within venous channels lined by endothelium. The diagnosis of Intravenous leiomyomatosis of the uterus without malignant transformation was retained. The patient was monitored for 14 months and subsequent computed tomography did not reveal any evidence of tumor recurrence. The follow-up will be performed annually till the age of menopause. Conclusion: Intravenous leiomyomatosis is a benign, rare and potentially lethal pathology. It especially affects premenopausal women with a history of uterine myoma, whether operated on or not. They require close and prolonged follow-up because of the high risk of recurrence.

Clinical analysis of 81 cases of intravenous leiomyomatosis confined to pelvic cavity

Objective: To investigate the clinical features, treatment and recurrence factors of intravenous leiomyomatosis (IVL) confined to the pelvic cavity after the operation. Methods: The clinical data of 81 patients who underwent surgery and were pathologically diagnosed with IVL at Women's Hospital, School of Medicine, Zhejiang University from January 2014 to March 2021, were analyzed retrospectively to explore the influencing factors of postoperative recurrence of IVL, including age, gravidity and parity, surgical methods, intraoperative conditions and so on. Results: (1) Clinical features: the age of 81 IVL patients was (43.9±8.1) years old; increased menstrual volume in 26 cases (32%, 26/81), prolonged menstrual period in 31 cases (38%, 31/81), frequency and urgency to urinate in 4 cases (5%, 4/81), abdominal pain and abdominal distension in 8 cases (10%, 8/81), and pelvic masses in 34 cases (42%, 34/81). IVL was diagnosed right in 4 of 72 patients (6%, 4/72) underwent preoperative ultrasound, right in 11 of 51 patients (22%, 11/51) underwent magnetic resonance imaging (MRI), and right in 4 of 19 patients (4/19) underwent CT. (2) Treatment: all patients with IVL underwent surgical treatment. Surgical procedure: myomectomy in 37 cases, total hysterectomy and bilateral salpingectomy in 19 cases, total hysterectomy and bilateral salpingo-ophorectomy in 25 cases. Surgical approach: hysteroscopic operation in 6 cases, transabdominal operation in 52 cases, laparoscopic operation in 23 cases. Fifty-three cases underwent rapid intraoperative pathological examination, 17 cases (32%, 17/53) of them were diagnosed right as IVL. (3) Influencing factors of IVL postoperative recurrence: among 81 patients with IVL, 3 cases were lost to follow-up, and 8 cases (10%, 8/78) had recurrence during follow-up. Age<35 years, number of pregnancies<2, number of births<2, number of fibroids ≥10, abnormal appearance of fibroids (long, vermicular, beadlike, cystic, etc.) and IVL invasion into adjacent vessels were all the risk factors influencing postoperative recurrence of IVL (all P<0.05). Methods of operation, ovariectomy or not, IVL invasion or not, maximum diameter of IVL, abundant blood vessels near uterine fibroids were not associated with postoperative recurrence of IVL (all P>0.05). Conclusions: The clinical manifestations and preoperative auxiliary examination of IVL are lack of specificity. Doctors need to pay attention to young patients with uterine fibroids, and choose the appropriate surgical scope when the characteristic manifestations of IVL be found during the operation, or the right diagnoses of IVL in the surgery's rapid intraoperative pathology be examined, and should remove the IVL lesions to reduce the recurrence as far as possible.

An Unusual Case

Intravenous leiomyomatosis (IVL) is a rare neoplasm characterized by histologically benign-looking smooth muscle cell tumor mass which grows within the intrauterine and extrauterine venous system. In this report, we present an unusual case of IVL which had originated from uterus and extended throughout the right cardiac chambers. A 36-year-old female patient came with a chief complaint of palpitation for 1-month duration. She also had a history of amenorrhea for 7 months. She had abdominal distension and doughy feeling of the abdomen. Seven months prior, ultrasound abdomen done during gynecological evaluation showed fibroid uterus. Echocardiography done showed multiple masses occupying almost all of the right atrium and moving in and out of the right ventricle. Inferior vena cava (IVC) was dilated and 90% of the IVC was filled with the mass. Cardiothoracic surgery and surgical oncology references were done. The patient was planned for simultaneous abdominal and cardiothoracic surgery. Large abdominal mass along with cardiac mass was resected along with mesentery, omentum, and abdominal lymph nodes. Histopathological and microscopic report was suggestive of leiomyoma and leiomyomatosis. Lymph node microscopy showed reactive hyperplasia.

Intravascular adenomyomatosis: a morphologic variant of intravenous leiomyomatosis associated with endometriosis and potential for misdiagnosis

Intravenous leiomyomatosis (IVL) is a quasi-malignant smooth muscle tumor involving lymphatic and venous spaces of the myometrium. Rare cases of IVL with admixed endometrial glands and stroma have been described, termed intravascular adenomyomatosis. We report four additional cases of intravascular adenomyomatosis and expand the clinicopathologic features of these rare tumors. Patients were 39-45 years old and presented with symptoms of dysmenorrhea, postmenopausal bleeding, or pelvic mass. All cases were associated with endometriosis. Three cases comprised intravascular bland smooth muscle tumors with plexiform features, and in some foci, the intravascular tumor contained endometrial type glands and stroma. In one case, there was extensive (>10) foci of intravascular adenomyomatosis without evidence of associated smooth muscle neoplasm but did have an endometrial polyp with adenomyomatous features. None of the cases had nuclear atypia, increased mitotic activity, or tumor cell necrosis. The endometrial stromal components were positive for CD10 and negative or weakly positive for desmin by immunohistochemistry. Two cases underwent molecular testing for JAZF1 and PHF1 rearrangements with negative results. Three patients had no evidence of disease at the time of the last follow-up, and one had persistent but stable disease 7 years after incomplete surgical removal and megestrol acetate treatment. Intravascular adenomyomatosis is a variant morphology rarely seen in IVL that lacks characteristic morphologic and molecular features of endometrial stromal sarcoma. Similar to IVL, prognosis is likely linked to completeness of surgical resection. In this study, we found that intravascular adenomyomatosis is frequently associated with endometriosis, a novel finding to add to the literature on this rare IVL variant.

Complete intravenous leiomyomatosis: a case report and literature review

Intravenous leiomyomatosis (IVL) is a relatively novel disease and can be aggressive. Since the first case was reported, it has a history of more than one hundred years, but the clinical incidence is extremely low, which has profound clinical research significance. Early recognition and management can prevent fatal consequences. IVL should be considered in female patients with a history of leiomyomas and intravascular filling defects. Because the patient's symptoms are not obvious, it is often misdiagnosed. We report on a female patient with IVL who presented with dizziness, and discuss the unexplained cause of filling defects within the right cardiac chambers and pulmonary arterial system. We present a complicated and rare case of IVL in a woman with a right atrial mass. A long, cord-like mass with inhomogeneous echo was detected by echocardiography. Coronary computed tomography (CT) showed a large hypodense mass occupying most of the right atrial cavity, spreading in both pulmonary arteries. Vascular ultrasound revealed a mixed echogenic mass invading the inferior vena cava like a rope. We performed surgery on her and complete resection on the tumor. We search for documents by searching databases such as National Center for Biotechnology Information (NCBI), China National Knowledge Infrastructure (CNKI) and Web of Science (WoS), etc. the recurrence rate recorded in the 4 cases series was 47.83% (11/23), 43.48% (20/46), 12.50% (2/16), and 10.00% (2/20), respectively.

Clinicopathologic features and clinical outcomes of intravenous leiomyomatosis of the uterus: A case series.

Rationale:Intravenous leiomyomatosis (IVL) is a rare and special type of smooth muscle tumor originating in the uterus. It is classified as a benign disease according to its histological features but shows the behavioral characteristics of a malignant tumor. It is easily misdiagnosed and recurrent. The purpose of this study was to retrospectively analyze clinicopathological data of 25 cases of IVL in order to enhance clinicians’ understanding of this rare disease.Patient concerns:We screened and identified 25 cases of IVL at our hospital from October 2013 to January 2020. Five patients had tumors.Diagnoses:The diagnosis in each case was pathologically confirmed after surgical treatment.Interventions:All patients were managed surgically. Although the surgical procedures were different, the surgical approach was geared towards achieving complete excision. Three patients received hormonal therapy with gonadotropinreleasing hormone agonists after surgery.Outcomes:We retrospectively reviewed all medical records and analyzed the clinicopathologic features and clinical outcomes of this disease as well as the correlations between the clinical features and risk of recurrence. Neither the symptoms nor the preoperative imaging results were suggestive of IVL in any of the cases. Except for two patients who were lost to follow-up, twenty-three patients who were followed up are still alive. Three patients experienced a recurrence.Lessons:The clinical manifestations and ultrasound images of IVL in the early stages are not typical; thus, IVL is easily misdiagnosed as uterine leiomyoma. Radiologists, pathologists, and surgeons should have a thorough understanding of IVL and a high index of vigilance for IVL in clinical practice. Surgery should always be aimed at achieving complete tumor excision. Patients with large lesions (≥7 cm) and lesions extending to the broad ligament may have an increased risk of recurrence. Early detection, diagnosis, and treatment are very important; once the diagnosis is confirmed, regular follow-ups are crucial.

MED12 exon 2 mutation is uncommon in intravenous leiomyomatosis: clinicopathologic features and molecular study

Intravenous leiomyomatosis (IVL) is a rare neoplasm that is characterized by smooth muscle cell proliferation within venous vessels. The aim of this study is to investigate the clinicopathological features, immunophenotypes, and MED12 gene mutations in IVL. Nine cases of IVL from the Affiliated Hospital of Qingdao University were collected, and the clinicopathological features were reviewed. The immunohistochemical expressions of p16, phosphatase and tensin homolog deleted on chromosome 10 (PTEN), alpha thalassemia/mental retardation syndrome X-linked (ATRX), retinoblastoma 1 (RB1), fumarate hydratase (FH), and p53, were evaluated. The mutation status of MED12 gene exon 2 was detected by Sanger sequencing. All the 9 patients were women ranging from 32 to 58 years, and uterine leiomyomas were identified in 5 patients. Immunohistochemical staining showed that all IVL and leiomyoma samples were positive for estrogen receptor and progesterone receptor, but negative for CD34. IVL displayed similar immunostaining patterns with their uterine counterparts with focal p16 immunostaining. FH, PTEN, ATRX, and RB1 were variably positive, and p53 and Ki-67 positive rates were less than 5% in all cases. Two novel genetic variations at MED12 exon 2, a synonymous mutation c.141C>T (p.Asn47=), and an in-frame deletion mutation c.133_147del15 (p.Phe45_Pro49del) were identified in two IVL cases. One missense mutation c.131G>A (p.Gly44Asp) was identified in one uterine leiomyoma. The remaining 11 tumor samples (7 IVL cases and 4 uterine leiomyomas) showed no mutations at MED12 exon 2. Our results showed two novel MED12 mutations in IVL. The MED12 mutations are different between IVL and uterine leiomyoma. These findings indicate that IVL is a unique entity and different from uterine leiomyoma.

Intravenous leiomyomatosis with intracardiac extension managed by one-stage surgical excision: a case presentation

BackgroundIntravenous leiomyomatosis is characterized by the extension of pathologically benign smooth muscle tumor into venous channels. Its extension into the heart is a very rare occurrence, which may lead to heart failure and occasionally sudden death. Due to its rarity, the condition is poorly understood and often misdiagnosed.Case presentationWe report a case of intravenous leiomyomatosis with intracardiac extension in a 40 years old woman who underwent hysterectomy for uterine leiomyoma (one year earlier), managed successfully through one-stage surgical approach with main focus on its imaging features.ConclusionThe diagnosis of intravenous leiomyomatosis should be considered in women who present with cardiac symptoms associated with a right atrial mass and who also have a pelvic mass or have previously undergone hysterectomy because of uterine leiomyoma.

Intravenous leiomyomatosis of the uterus: A clinicopathologic analysis of 13 cases with an emphasis on histogenesis

The object of this study is to investigate the prognosis and potential histologic origin of uterine intravenous leiomyomatosis (IVL). We retrospectively analyzed the clinicopathologic and immunohistochemical features of 13 cases of IVL from a single institute. The patients underwent hysterectomy (3 with bilateral salpingo-oophorectomy, and 8 with excision of the broad ligaments and/or parametrium). They survived with no evidence of disease for 6–90 months. Intravascular worm-like plugs were macroscopically identified in the myometrium in 10 of 13 cases (76.9%). Six patients (46.1%) had extra-uterine involvement. Histopathologically, the intravascular tumor foci were covered with endothelium. We found the presence of congested vessels in the tumor and the surrounding myometrium in 9 of 13 cases (69.2%). The concurrent entities included seven uterine leiomyomas, one leiomyoma with adenomyosis, one adenomyosis, and one pulmonary benign metastasizing leiomyoma. Estrogen receptor and desmin were positive in IVL, and negative in the vascular wall. We conclude that IVL confined to the uterus may have a favorable prognosis, and we believe IVL is likely to originate from contiguous leiomyoma or myometrium.

Intravascular Leiomyomatosis and the CT Appearance: A Case Report and Review of Literature

Intravenous leiomyomatosis (IVL) is a benign smooth muscle tumor which may cause fatal outcome. Furthermore, the clinical manifestations are variable and nonspecific, typically resulting in misdiagnosis. Computerized Tomography (CT) played a vital role in the diagnostic process and pre-surgical assessment. Here we describe one case of IVL extending from the inferior vena cava to the right atrium and ventricle, then review the literature and discuss the imaging findings and clinical manifestation of this tumor. It is suggestive for clinicians to be aware of this condition and highly suspect a middle-aged woman with a history of uterine leiomyoma presenting with an inferior vena cava filling defect or mass shadow to early diagnose and treat.

Intravenous leiomyomatosis with right atrium extension in two patients: A case report.

Intravenous leiomyomatosis (IVL) is a rare benign tumor of the uterus mesoderm, which can spread via the vein, from the micro-vein to the inferior vena cava, and even to the right atrium. IVL was first described this disease in 1896 and the first presented case of IVL with right atrium extension in the autopsy was described in 1907. On rare occasions, the tumors may extend into the regional and systemic veins, thus reaching the heart. This may subsequently cause intracardiac leiomyomatosis (ICL), which may lead to congestive heart failure and on occasion, sudden fatalities. Due to its rarity and diffuse symptoms, the misdiagnosis of ICL is common and as a result, the condition may be under-reported. The present study reported two cases of IVL resected at the General Hospital of Shenyang Military Command. A hysteromyomectomy for uterine fibroids was performed on each patient (Case 1, 41 years ago; Case 2, 3 years ago). One patient presented with chest pain following a period of activity and the other presented with heart neoplasm during a routine health examination.

Intravenous leiomyomatosis with intracardiac extension depicted on computed tomography and magnetic resonance imaging scans: A report of two cases and a review of the literature.

Intravenous leiomyomatosis (IVL) is defined as a benign smooth muscle tumor, growing within systemic veins. IVL with intracaval and intracardiac extension has rarely been reported in radiological and oncological journals. The present study describes 2 cases of IVL extending from the inferior vena cava to the right atrium and ventricle, and discusses the imaging findings and differential diagnosis of this tumor entity. The two patients, who complained of palpitations, shortness of breath or syncope, were surgically treated, with complete resection of the cardiac and intracaval tumors. Pathological examinations were suggestive of IVL. The postoperative course of the two patients was uneventful, and no signs of recurrence were observed on follow-up. Computed tomography and magnetic resonance imaging played a vital role in the diagnostic process and presurgical assessment. The results of the present study indicate that IVL should be considered upon presentation of a soft mass in systemic veins, even when the mass extends to the right cardiac chambers, in female patients, particularly in patients with a history of uterine myoma.

Intravascular leiomyomatosis

Intravenous leiomyomatosis is a rare disease from a group of tumors with the indefinite grading potential. The paper describes two cases of intravenous leiomyomatosis with its detailed morphological pattern, molecular genetic findings, and a brief literature review. Losses of heterozygosity of microsatellite repeats thatwere located on chromosome 10 in 10q22.1 and common in uterine leiomyosarcomas were found in both cases. Investigations of the morphological and biological characteristics of leimyomatosis are important to clarify the key molecular mechanisms underlying the development of this nosological entity and to determine etiopathogenetic relationships between intravenous leiomyomatosis and other uterine smooth muscle neoplasms.

Huge Intravascular Tumor Extending to the Heart: Leiomyomatosis.

Intravenous leiomyomatosis (IVL) is a rare neoplasm characterized by histologically benign-looking smooth muscle cell tumor mass, which is growing within the intrauterine and extrauterine venous system. In this report we aimed to present an unusual case of IVL, which is originating from iliac vein and extended throughout to right cardiac chambers. A 49-year-old female patient, who was treated with warfarin sodium due to right iliac vein thrombosis, was admitted to our department with intermittent dyspnea, palpitation, and dizziness. Physical examination was almost normal except bilateral pretibial edema. On magnetic resonance venography, there was an intravenous mass, which is originated from right internal iliac vein and extended into the inferior vena cava. Transthoracic echocardiography and transesophageal echocardiography revealed a huge mass extending from the inferior vena cava through the right atrium, with obvious venous occlusion. Thoracic, abdominal, and pelvic MR showed an intravascular mass, which is concordant with leiomyomatosis. Surgery was performed through median sternotomy. A huge mass with 25-cm length and 186-gr weight was excised through right atrial oblique incision, on beating heart with cardiopulmonary bypass. Histopathologic assessment was compatible with IVL. Exact strategy for the surgical treatment of IVL is still controversial. We used one-stage approach, with complete resection of a huge IVL extending from right atrium to right iliac vein. In such cases, high recurrence rate is a significant problem; therefore it should be kept in mind.

Recurrent chromosomal aberrations in intravenous leiomyomatosis of the uterus: high-resolution array comparative genomic hybridization study.

Uterine intravenous leiomyomatosis (IVL) is a distinct smooth muscle neoplasm with a potential of clinical aggressiveness due to its ability to extend into intrauterine and extrauterine vasculature. In this study, chromosomal alterations analyzed by oligonucleotide array comparative genomic hybridization were performed in 9 cases of IVL. The analysis was informative in all cases with multiple copy number losses and/or gains observed in each tumor. The most frequent recurrent loss of 22q12.3-q13.1 was observed in 6 tumors (66.7%), followed by losses of 22q11.23-q13.31, 1p36.13-p33, 2p25.3-p23.3, and 2q24.2-q32.2 and gains of 6p22.2, 2q37.3 and 10q22.2-q22.3, in decreasing order of frequency. Copy number variants were identified at 14q11.2, 15q11.1-q11.2, and 15q26.2. Genes mapping to the regions of loss include CHEK2, EWS, NF2, PDGFB, and MAP3K7IP1 on chromosome 22q, HEI10 on chromosome 14q, and succinate dehydrogenase subunit B, E2F2, ARID1A KPNA6, EIF3S2 , PTCH2, and PIK3R3 on chromosome 1p. Regional losses on chromosomes 22q and 1p and gains on chromosomes 12q showed overlaps with those previously observed in uterine leiomyosarcomas. In addition, presence of multiple chromosomal aberrations implies a higher level of genetic instability. Follow-up polymerase chain reaction (PCR) sequencing analysis of MED12 gene revealed absence of G> A transition at nucleotides c.130 or c.131 in all 9 cases, a frequent mutation found in uterine leiomyoma and its variants. In conclusion, this is the first report of high-resolution, genome-wide investigation of IVL by oligonucleotide array comparative genomic hybridization. The presence of high frequencies of recurrent regional loss involving several chromosomes is an important finding and likely related to the pathogenesis of the disease.

Intracardiac extension of intravenous leiomyomatosis in a woman with previous hysterectomy and bilateral salpingo-oophorectomy: A case report and review of the literature

Intravenous leiomyomatosis (IVL) is a rare tumor, characterized by benign smooth muscle growth inside veins. The tumor arises from the uterine venous wall or uterine leiomyomas and is usually confined to the pelvic cavity. However, on rare instances, it may extend into the cardiac cavity (Pathol Annu 1988;23 Pt 2:153–158), and the pulmonary system (Arch Gynecol Obstet 2001;264:209–210). Treatment consists of surgical removal of the tumor, cessation of ovarian function and avoidance of estrogen replacement therapy (Gynecol Obstet Invest 2004;58:168–170). We present a case of intravenous leiomyomatosis with extension from IVC to RA, RV and PA, with an unusually rapid course of progression in the absence of estrogen (TAH-BSO, without concomitant hormonal therapy).

Clinicopathologic features of intravenous leiomyomatosis with intracardiac extension

To investigate clinicopathological features, immunophenotype and differential diagnosis of intravenous leiomyomatosis with intracardiac extension. Clinical manifestations, morphologic features, and immunohistochemical staining were retrospectively analyzed in 19 cases of intravenous leiomyomatosis with intracardiac extension. The patients' age ranged from 33 to 59 years (mean 44 years). Clinical presentation included chest tightness, palpitation, dyspnea, edema of low extremity, abdominal distention or hypermenorrhea. However, a few patients were asymptomatic. Grossly, intravenous leiomyomatosis in most cases demonstrated coiled or nodular growth within the myometrium with worm-like involvement of the uterine vein in broad ligament or other pelvic veins with continued extension into the vena cava and the heart. The intravenous tumor surface was generally smooth and rubbery, with a greyish-white color. Microscopically, the tumors were composed of spindle cells with rare mitotic figures and the presence of abundant thick-walled vessels. Marked fibrosis, hyalinization, myxoid and edematous changes were common. The tumor cells were positive for SMA, ER, PR and desmin but negative for HMB45 and S-100. CD10 and CD34 were positive in 4 patients and negative in the remaining cases. Intravenous leiomyomatosis with intracardiac extension is a rare disease among women of child-bearing age, with specific morphologic manifestations and immunohistochemical profiles.

Heart extension of an intravenous leiomyomatosis

Intravenous leiomyomatosis is a rare growth pattern of benign uterine leiomyomas. Approximately 300 cases of intravenous leiomyomatosis have been reported in the English literature; however, there have only been a few in radiology journals. Even more rare is its extension through the inferior vena cava and heart chambers. Radiologic diagnosis can be challenging, as it is often difficult to differentiate it from other entities. We present a case of a 45-year-old woman with a cardiac extension of an intravenous leiomyomatosis and discuss the cross-sectional imaging appearance of this entity as well as the differential diagnosis.